Cognitive and speech-language performance in children with ataxia telangiectasia
SourceDevelopmental Neurorehabilitation, 14, 5, (2011), pp. 315-322
Article / Letter to editor
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Paediatrics - OUD tm 2017
SubjectDCN 2: Functional Neurogenomics; N4i 4: Auto-immunity, transplantation and immunotherapy; NCEBP 10: Human Movement & Fatigue DCN 1: Perception and Action; NCEBP 8: Psychological determinants of chronic illness; NCEBP 8: Psychological determinants of chronic illness DCN 1: Perception and Action; NCEBP 10: Human Movement & Fatigue DCN 1: DCN 1: Perception and Action
OBJECTIVE: To describe cognitive and speech-language functioning of patients with ataxia-telangiectasia (A-T) in relation to their deteriorating (oculo)motor function. DESIGN: Observational case series. METHODS: Cognitive functioning, language, speech and oral-motor functioning were examined in eight individuals with A-T (six boys, two girls), taking into account the confounding effects of motor functioning on test performance. RESULTS: All patients, except the youngest one, suffered from mild-to-moderate/severe intellectual impairment. Compared to developmental age, patients showed cognitive deficits in attention, (non)verbal memory and verbal fluency. Furthermore, dysarthria and weak oral-motor performance was found. Language was one of the patients' assets. CONCLUSION: In contrast to the severe deterioration of motor functioning in A-T, cognitive and language functioning appeared to level off with a typical profile of neuropsychological strengths and weaknesses. Based on our experiences with A-T, suggestions are made to determine a valid assessment of the cognitive and speech-language manifestations.
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